成人噬血细胞性淋巴组织细胞增生症患者在重症监护病房的并发症及预后：一项16例病例的回顾性研究
Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases.
作者信息
Kapoor Sumit, Morgan Christopher K, Siddique Muhammad Asim, Guntupalli Kalpalatha K
机构信息
Department of Critical Care Medicine, Montefiore Medical Center, Bronx, NY 10467, United States.
Department of Pulmonary, Critical Care and Sleep, Baylor College of Medicine, Houston, TX 77030, United States.
出版信息
World J Crit Care Med. 2018 Nov 30;7(6):73-83. doi: 10.5492/wjccm.v7.i6.73.
AIM
To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis (HLH) in the intensive care unit (ICU).
METHODS
We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.
RESULTS
Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years. Median age of presentation was 49 years and 10 (63%) were males. Median Sequential Organ Failure Assessment (SOFA) score at the time of ICU admission was 10. Median ICU length of stay (LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in (88%) patients, followed by acute kidney injury (81%) and acute respiratory failure requiring mechanical ventilation (75%). Nine patients (56%) developed disseminated intravascular coagulation and eight (50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed. Multi system organ failure was the most common cause of death seen in 12 (75%) patients. The 30 d mortality was 37% (6 cases) and 90 d mortality was 81% (13 cases). There was no difference in mortality based on age (above or less than 50 years), SOFA score on ICU admission (more than or less than 10), immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.
CONCLUSION
HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.
目的
研究入住重症监护病房（ICU）的噬血细胞性淋巴组织细胞增生症（HLH）成年患者的管理、并发症及预后情况。
方法
我们对2013年1月1日至2017年6月30日期间入住贝勒医学院两所学术性医疗ICU且诊断为“HLH”的成年患者进行了一项回顾性观察研究。HLH采用组织细胞协会提出的HLH-2004标准进行诊断。
结果
4年间有16例成年HLH患者入住医疗ICU。发病的中位年龄为49岁，10例（63%）为男性。ICU入院时序贯器官衰竭评估（SOFA）评分的中位数为10分。ICU住院时间（LOS）的中位数为11.5天，住院总时间的中位数为29天。脓毒性休克和急性呼吸衰竭是需要入住ICU的主要诊断原因。脓毒性休克是（88%）患者中最常见的ICU并发症，其次是急性肾损伤（81%）和需要机械通气的急性呼吸衰竭（75%）。9例（56%）患者发生弥散性血管内凝血，8例（50%）出现急性肝衰竭。观察到10次具有临床意义的出血事件。多系统器官衰竭是最常见的死亡原因，12例（75%）患者因此死亡。30天死亡率为37%（6例），90天死亡率为81%（13例）。基于年龄（50岁以上或以下）、ICU入院时的SOFA评分（大于或小于10分）、免疫抑制、诊断HLH的时间或直接入住ICU与从病房转入ICU，死亡率无差异。
结论
HLH是一种破坏性疾病，在ICU中预后较差。对于脓毒性休克/多系统器官衰竭且伴有进行性双系/全血细胞减少且对ICU标准治疗无反应的患者，重症监护医生需要对HLH有高度的临床怀疑。
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